Bottom Line:
No associated disorder or presumed cause was found in any case.Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5).Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes.

Affiliation: Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

ABSTRACT

Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported group of disorders characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes. We report five Japanese cases fulfilling the criteria of IPPFE and address whether it should be considered a separate clinicopathologic entity. And this study was an attempt to identify features in common between IPPFE and previously described idiopathic upper lobe fibrosis (IPUF), allowing IPPFE to be considered as a distinct entity in our Japanese series.

Results: There were four males and one female, aged 70±2.76 yr. No associated disorder or presumed cause was found in any case. Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5). Chest X-ray showed marked apical pleural thickening in all cases. Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes. In all cases in this study, markedly thickened visceral pleura and prominent subpleural fibrosis characterized by both elastic tissue and dense collagen were clearly shown. All cases were alive at the last follow-up, 17.6±13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically.

Conclusions: IPPFE deserves to be defined as a separate, original clinicopathologic entity owing to its uniformity and IPPFE has some features in common with previously described idiopathic upper lobe fibrosis (IPUF). Our limited experience with a cohort of 5 subjects suggests that IPPFE can be rapidly progressive.

Figure 4: Clinical Course of the cases. Case 1 radiologically deteriorated a year after diagnosis (a, b). Other cases present similar behavior. (c) Decrease in FVC was confirmed in all cases during the follow up. The median follow up was 12.1 (range 4.37-22.2) months.

Mentions:
All cases initially had no treatment. The mean duration of follow-up after the pathologic diagnosis was 12.1 months (range 4.4 - 22). The total duration of follow-up from the onset of the symptoms to the last follow-up was 45.2 months (range 7–83). All patients were alive at the last follow-up (Table2), but all had disease progression according to clinical and lung function tests, especially in terms of %FVC (Figure4).

Figure 4: Clinical Course of the cases. Case 1 radiologically deteriorated a year after diagnosis (a, b). Other cases present similar behavior. (c) Decrease in FVC was confirmed in all cases during the follow up. The median follow up was 12.1 (range 4.37-22.2) months.

Mentions:
All cases initially had no treatment. The mean duration of follow-up after the pathologic diagnosis was 12.1 months (range 4.4 - 22). The total duration of follow-up from the onset of the symptoms to the last follow-up was 45.2 months (range 7–83). All patients were alive at the last follow-up (Table2), but all had disease progression according to clinical and lung function tests, especially in terms of %FVC (Figure4).

Bottom Line:
No associated disorder or presumed cause was found in any case.Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5).Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes.

Affiliation:
Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

ABSTRACT

Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported group of disorders characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes. We report five Japanese cases fulfilling the criteria of IPPFE and address whether it should be considered a separate clinicopathologic entity. And this study was an attempt to identify features in common between IPPFE and previously described idiopathic upper lobe fibrosis (IPUF), allowing IPPFE to be considered as a distinct entity in our Japanese series.

Results: There were four males and one female, aged 70±2.76 yr. No associated disorder or presumed cause was found in any case. Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5). Chest X-ray showed marked apical pleural thickening in all cases. Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes. In all cases in this study, markedly thickened visceral pleura and prominent subpleural fibrosis characterized by both elastic tissue and dense collagen were clearly shown. All cases were alive at the last follow-up, 17.6±13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically.

Conclusions: IPPFE deserves to be defined as a separate, original clinicopathologic entity owing to its uniformity and IPPFE has some features in common with previously described idiopathic upper lobe fibrosis (IPUF). Our limited experience with a cohort of 5 subjects suggests that IPPFE can be rapidly progressive.